| Efficacy of prophylaxis and genotype-phenotype correlation in patients with severe Factor X deficiency in Iran. | |
| | |
MedLine Citation:
|
PMID: 21854511 Owner: NLM Status: Publisher |
Abstract/OtherAbstract:
|
Summary. We aimed to evaluate the effect of regular prophylaxis with a Factor X (FX) concentrate for patients with severe FXD in Iran and to assess the correlation of the genotype and phenotype in these patients. Ten patients with severe FXD (FX activity <1%) were enrolled and characterized during 2010-2011. Prophylaxis with 20 IU FX P Behring per kg body weight was administered once a week. FX levels, were monitored at baseline, 15 and 30 min, 1, 3, 6, 12, 24, 48, 72 and 96 h after starting prophylaxis. All patients were followed for 1 year. The mean age of the patients was 15 ± 7.8 years (age range of: 6-27 years). One patient had anaphylactic reaction after the first infusion, and the treatment was stopped. During one-year follow-up after starting prophylaxis, no bleeding symptoms occurred in any patient who tolerated and remained on the prophylaxis programme and all of them had a FX level of 1% or above. The maximum level of FX activity has been observed at 15 min after starting prophylaxis. A level of 1.5-3.5% was detected after 96 h. Homozygous mutations p.Arg40Thr (Arg-1Thr), p.Gly51Arg and p.Glu69Lys were detected in patients with intracranial haemorrhage. In our patients, significant decrease in symptoms without any complication after administration of FX, was demonstrated in all except one patient who had an anaphylactic reaction. It seems that the dose of 20 IU kg(-1) could be probably the best choice for patients with severe FXD, who require regular prophylaxis. |
| | |
Authors:
|
M Karimi; A Vafafar; S Haghpanah; M Payandeh; P Eshghi; H Hoofar; A Afrasiabi; J Gerdabi; R Ardeshiri; M Menegatti; F Peyvandi |
Publication Detail:
|
Type: JOURNAL ARTICLE Date: 2011-8-19 |
Journal Detail:
|
Title: Haemophilia : the official journal of the World Federation of Hemophilia Volume: - ISSN: 1365-2516 ISO Abbreviation: - Publication Date: 2011 Aug |
Date Detail:
|
Created Date: 2011-8-22 Completed Date: - Revised Date: - |
Medline Journal Info:
|
Nlm Unique ID: 9442916 Medline TA: Haemophilia Country: - |
Other Details:
|
Languages: ENG Pagination: - Citation Subset: - |
Copyright Information:
|
© 2011 Blackwell Publishing Ltd. |
Affiliation:
|
Hematology research center, Shiraz University of Medical Sciences, Shiraz, Iran Hemophilia Treatment Center, Kermanshah University of Medical Sciences, Kermanshah, Iran Hemophilia Treatment Center, Mophid Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran Hemophilia Treatment Center, Isfahan University of Medical Sciences, Isfahan, Iran UOS Dipartimentale per la Diagnosi e la Terapia delle Coagulopatie. A. Bianchi Bonomi Hemophilia and Thrombosis Center, Fondazione IRCCS Cà Granda Ospedale Maggiore Policlinico, Università degli Studi di Milano and Luigi Villa Foundation, Milan, Italy. |
Export Citation:
|
APA/MLA Format Download EndNote Download BibTex |
| MeSH Terms | |
Descriptor/Qualifier:
|
|
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
Previous Document: Liver transplantation in the haemophilia patient.
Next Document: New Exoelectrogen Citrobacter sp. SX-1 Isolated from a Microbial Fuel Cell.